Von Hippel-Lindau (VHL) syndrome is characterized by a range of tumors including phaeochromocytomas, pancreatic adenomas, cerebellar haemangioblastomas, and renal cell carcinomas. A 50-year-old male presented with a three-week history of headache. Additionally, the patient exhibited signs of hypertension. Ultrasonography (USG) abdomen and pelvis showed a solid mass lesion in the left adrenal gland, iso-echoic to the renal cortex. On contrast-enhanced computed tomography (CECT) of the brain, a well-defined solid cystic lesion was seen in the left posterior cerebellar hemisphere. Small nodular enhancing lesions were seen in the right cerebellar hemisphere. On further imaging with MRI brain contrast, the lesions in the cerebellum were diagnosed as multifocal hemangioblastomas. Laboratory investigations revealed elevated urinary metanephrines and normetanephrine, suggesting pheochromocytoma. Based on radiological and biochemical investigations, with the features of cerebellar haemangioblastomas and pheochromocytoma, a diagnosis of VHL syndrome was made.

von Hippel-Lindau (VHL) syndrome (OMIM #193300) is an autosomal dominant disorder with incomplete penetrance occurring due to a mutation in the VHL gene present on chromosome 3. We present the case of a 21-year-old male with a history of retinoblastoma presenting with intermittent headaches for one month. He was a known hypertensive and his blood pressure on presentation was 180/100 mmHg. A secondary cause for his hypertension was sought. Multiple cysts in his pancreas, both his kidneys, and a mass in the right suprarenal fossa were detected on an abdominal ultrasonogram and a subsequent computed tomography scan of the abdomen. VHL and a pheochromocytoma were suspected, and a positron emission tomography-computed tomography scan was done which collaborated with the above findings. The presence of multiple cystic lesions in the pancreas and kidneys, especially in an individual with a family history of VHL syndrome, should alert the physician to the possibility of VHL syndrome. The need for evaluation of causes for hypertension, especially in young individuals with resistant hypertension, is also highlighted.

Pheochromocytomas are tumors that develop from the chromaffin cells of the adrenal medulla. More than 40% of cases of pheochromocytomas are associated with genetic conditions such as neurofibromatosis type 1 (NF1) or von Hippel-Lindau syndrome. Cystic pheochromocytomas are rare, generally asymptomatic, and thus of bigger size at the time of diagnosis. Surgical treatment is necessary to prevent cardiovascular morbidity and malignancy risk. We report the case of a 27-year-old patient admitted for further examination of a left adrenal mass that was discovered by an abdominal CT scan in the context of abdominal pain associated with hypertension evolving for three years. The clinical examination showed the presence of multiple café au lait spots, axillary and inguinal freckling with two dermal neurofibromas diagnosed clinically, as well as Lisch nodules on bilateral ophthalmic examination, thus meeting the clinical criteria for the diagnosis of NF1. The clinical laboratory investigation showed elevated urinary metanephrine and normetanephrine levels. CT scan examination showed a 10 cm left adrenal cystic mass on abdominal CT. This mass uptake of the radioligand in metaiodobenzylguanidine (MIBG) scintigraphy without secondary extra-adrenal localization allowed the diagnosis of a seemingly benign cystic pheochromocytoma to be made. The patient was put on presurgical drug preparation with volume expansion and then underwent left unilateral adrenalectomy. The histopathological study was in favor of a rather aggressive cystic pheochromocytoma with a pheochromocytoma of the adrenal gland scaled (PASS) score of 9. Blood pressure and urine catecholamines at seven days, three months, six months, and one year after surgery were normalized. Cystic pheochromocytoma is a rare tumor with a potentially poor prognosis. It is characterized by a more insidious evolution and a larger volume at diagnosis. It should be considered a diagnosis in patients with a cystic adrenal mass or an extra-adrenal mass with fluctuating blood pressure during surgery. This case illustrates the importance of both presurgical preparation and screening for pheochromocytoma in neurofibromatosis type 1.

There is no consensus on whether intravenous rehydration must be added after preoperative phenoxybenzamine (PXB) administration for pheochromocytoma. The aim of this study is to investigate whether abandonment of intravenous volume expansion after PXB administration is associated with intraoperative hemodynamic instability.
83 Patients with pheochromocytoma received surgical treatment in the Department of Urology, Handan First Hospital, between October 2014 and July 2022. All patients were subclassified into either the hemodynamic stability group (HS group) or the hemodynamic instability group (HU group) according to whether intraoperative hemodynamic instability occurred, with 51 cases in HS group and 32 cases in HU group. Differences in data between the two groups were examined, and the risk factors for intraoperative hemodynamic instability were analyzed using logistic regression.
The results of the analysis showed no statistically significant differences in age, sex, location of the tumor, surgical method, body mass index (BMI) ≥ 24 kg/m2, blood and urine catecholamine test results, preoperative oral PXB followed by combined intravenous volume expansion, proportion of patients with hypertension or diabetes mellitus or coronary heart disease between the two groups (P>0.05). The size of the tumor in the HS group was smaller than that in the HU group (5.3 ± 1.9 cm vs 6.2 ± 2.4 cm P=0.010). Multivariate analyses demonstrated that abandonment of intravenous volume expansion after preoperative receipt of α-blockers in patients with adrenal pheochromocytoma was not an independent risk factor for intraoperative hemodynamic instability. Only the tumor size (P=0.025) was an independent risk factor for intraoperative hemodynamic instability.
The purpose of general preoperative intravenous fluid expansion is to prevent hypotension after the tumor has been resected. In the current study, we indicated that preoperative management of pheochromocytomas using the α-blocker PXB in combination with intravenous volume expansion does not further reduce the risk of intraoperative hemodynamic instability or postoperative complications compared with oral PXB alone. Therefore, our study supports preoperative management of pheochromocytoma with a single α-blocker, PXB, as sufficient.

Radiomics is a promising research field that combines big data analysis (from tissue texture analysis) with clinical questions. We studied the application of CT texture analysis in adrenal pheochromocytomas (PCCs) to define the correlation between the extracted features and the secretory pattern, the histopathological data, and the natural history of the disease. A total of 17 patients affected by surgically removed PCCs were retrospectively enrolled. Before surgery, all patients underwent contrast-enhanced CT and complete endocrine evaluation (catecholamine secretion and genetic evaluation). The pheochromocytoma adrenal gland scaled score (PASS) was determined upon histopathological examination. After a resampling of all CT images, the PCCs were delineated using LifeX software in all three phases (unenhanced, arterial, and venous), and 58 texture parameters were extracted for each volume of interest. Using the Mann-Whitney test, the correlations between the hormonal hypersecretion, the malignancy score of the lesion (PASS > 4), and texture parameters were studied. The parameters DISCRETIZED_HUpeak and GLZLM_GLNU in the unenhanced phase and GLZLM_SZE, CONVENTIONAL_HUmean, CONVENTIONAL_HUQ3, DISCRETIZED_HUmean, DISCRETIZED_AUC_CSH, GLRLM_HGRE, and GLZLM_SZHGE in the venous phase were able to differentiate secreting PCCs (p < 0.01), and the parameters GLZLM_GLNU in the unenhanced phase and GLRLM_GLNU and GLRLM_RLNU in the venous differentiated tumors with low and high PASS. CT texture analysis of adrenal PCCs can be a useful tool for the early identification of secreting or malignant tumors.

Background Open adrenalectomy is an invasive surgical procedure that is commonly performed for adrenal gland neoplasms in developing countries. Due to its complexity, the patients are predisposed to a number of complications and dismal outcomes. The objective of our study is to assess different characteristics of patients undergoing open adrenalectomy, including their histology, postoperative complications, and outcomes. Methods This retrospective cross-sectional study included 107 patients undergoing open adrenalectomy for primary adrenal gland neoplasms. Patients with bilateral involvement, metastatic disease, or unresectable tumors were excluded. Patients were evaluated for different features that included demographic data, tumor properties, postoperative outcomes, and complications. Results Out of 107 patients, 45 (42.1%) were females. The mean age of the patients was 47.53 ± 8.45 years. Abdominal pain and severe headaches were the most common presenting complaints. A total of 96 (89.7%) tumors were benign, while 11 (10.3%) were malignant. Upon the histopathological examination of the resected specimen, adrenal adenoma was present in 49 (45.8%) cases, while adrenal pheochromocytoma was present in 41 (38.3%) cases. A total of 51 patients developed different postoperative complications including surgical site infections (22.4%), atelectasis (11.2%), deep venous thrombosis (7.5%), and retroperitoneal hematoma (5.6%). In-hospital mortality occurred in three (2.8%) patients. Conclusion Surgical site infections, atelectasis, deep venous thrombosis, and retroperitoneal hematoma were frequent postoperative complications after open adrenalectomy. These complications increase morbidity and mortality, especially in developing countries. Improved surgical techniques, intraoperative hemostasis, and multidisciplinary approach can yield favorable postoperative outcomes.

BACKGROUND: Malignant pheochromocytomas are rare endocrine tumors that develop within chromaffin tissue. The diagnosis of malignancy is based on neoplastic recurrence or the presence of metastasis in organs that lack chromaffin tissue. We report a series of four cases because of their diagnostic and therapeutic particularities.
METHODS: we describe four clinical cases of patients with malignant pheochromocytoma whose Menard triad \"headache-palpitations-sweating\" was present in three out of four patients, the methoxylated derivatives were measured in 4 patients, 3 of which had high values, all of our patients carried out a CT scan which objectified signs of malignancy, MRI was performed on a single patient; presenting with a neoplastic recurrence; looking for a locoregional invasion.
CONCLUSIONS: Pheochromocytoma (PC) is a rare neuroendocrine tumor derived from the chromaffin cells of the adrenal medulla. Its annual incidence is 2 to 8 per million adults. A peak frequency is observed between 30 and 40 years of age. Approximately 10% of pheochromocytomas are malignant and in 10% of cases, bilateral localization is observed. Criteria for malignancy include the invasion of neighboring organs, a large tumor, the presence of lymphadenopathy on imaging, or fixation on scintigraphy. Surgery for MAP is not always curative. In the case of multiple liver metastases, treatment is based on adrenalectomy, which can be effectively combined with chemoembolization, cryoablation, or radiofrequency techniques.
CONCLUSIONS: The main prognostic factors of the malignant pheochromocytomas are a large tumor volume, the existence or number of visceral metastases, and the presence of a mutation in the SDHB (Succinate dehydrogenase B) gene.

A total of 17 (10 adrenal and 7 extraadrenal) pheochromocytomas were examined immunohistochemically for the localization of catecholamine-synthesizing enzymes-tyrosine hydroxylase (TH), dopamine β-hydroxylase (DBH), and phenylethanolamine N-methyltransferase (PNMT)-as well as the marker peptides for pheochrornocytomas (i.e., met-enkephalin-arggly-leu [MEAGL]). Normal adult, fetal, and newborn human adrenal medullas were also examined. Six adrenal pheochromocytomas showed immunohistochernically positive staining for PNMT; 5 of these cases demonstrated elevated serum adrenalin levels, This indicated morphofunctional correlation. PNMT-positive cells were mostly positive for TH but did not show co-localization of MEAGL except in 1 case, Absence of co-localization was considered to reflect the physiological condition of the specimen, based on the similar staining in the normal adult adrenal medulla. Primary culture and immunoelectron microscopy suggested the processing and synthesis of adrenalin and MEAGL in the secretory granules.Seven extraadrenal pheochromocytornas were negative for PNMT and suggested the lack of adrenalin synthesis.

Since flavonols are antioxidant agents, they could in principle, beneficially affect neurodegenerative diseases where reactive oxygen species are involved. Quercetin derivatives are the most abundant dietary flavonoids, and we have investigated the capacity of quercetin-3-O-β-d-glucopyranoside (Q3G) isolated from Echinophora cinerea to protect PC12 cells from H2O2-induced cytotoxicity. Direct cytotoxic effects of H2O2 on PC12 in presence and absence of Q3G were evaluated. H2O2 induced cytotoxicity in a concentration dependent manner (IC50=118 ± 5.09 μM, 24h). Pretreatment of cells with non-toxic concentrations of Q3G protected cells from H2O2-induced cytotoxicity, leading to a decrease in the generation of reactive oxygen species. These observations qualify Q3G as an interesting dietary compound worth further investigation as a cytoprotective agent.

BACKGROUND: Spontaneous rupture of adrenal pheochromocytoma is an extremely rare condition that can be lethal. Optimal treatment in these cases is still unclear.
METHODS: We conducted a comprehensive review of medline articles on ruptured pheochromocytomas to locate all research done on this topic. Based on the literature review and one additional case at European Gaza Hospital, we analyzed clinical presentations, hemorrhage site, tumour side, mortality rate, and treatment options.
RESULTS: In addition to our case, we identified 53 cases of ruptured pheochromocytoma. A review of all 53 cases revealed that 17 (32%) of the patients died, and that no mortality occurred among the 12 patients who received an alpha-blocker (to control high blood pressure) and fluid infusion therapy before surgery. Only 3 patients, including our case, underwent elective surgery after transcatheter arterial embolization (tae).
CONCLUSIONS: Surgical treatment should be considered for ruptured pheochromocytoma. Surgical approaches involve either emergency or elective surgery. It has been reported that emergency surgery is commonly associated with a high mortality rate; no deaths were reported in patients who underwent elective surgery. We therefore consider that, if a patient has hemodynamic instability, tae can be an effective and a safe procedure for achieving hemostasis and maintaining the patient in good condition until surgery can be performed.